Acute rheumatic fever continues to cause a large burden of mortality and morbidity in developing countries. It is less common in developed countries but continues to be seen in indigenous communities and during outbreaks.
No single test can diagnose acute rheumatic fever. Diagnosis is clinical and relies on the Jones criteria.
The 5 major manifestations of acute rheumatic fever are carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules, of which the most common are carditis and arthritis.
The Jones criteria were revised in 2015 to include separate criteria for low-risk and moderate- to high-risk populations.
While all other manifestations of acute rheumatic fever resolve without sequelae, carditis can lead to chronic rheumatic heart disease.
No treatment has been shown to alter the progression of acute rheumatic fever to chronic rheumatic heart disease.
Secondary prophylaxis can improve the prognosis of established rheumatic valvular disease.
Acute rheumatic fever is an autoimmune disease that may occur following group A streptococcal throat infection. It can affect multiple systems, including the joints, heart, brain, and skin. Only the effects on the heart can lead to permanent illness; chronic changes to the heart valves are referred to as chronic rheumatic heart disease. Without long-term penicillin secondary prophylaxis, acute rheumatic fever can recur, leading to cumulative damage to the cardiac valvular tissue.
History and exam
Key diagnostic factors
- joint pain
Other diagnostic factors
- recent sore throat or scarlet fever
- chest pain
- shortness of breath
- heart murmur
- pericardial rub
- signs of cardiac failure
- swollen joints
- emotional lability and personality changes
- jerky, uncoordinated choreiform movements
- inability to maintain protrusion of the tongue
- milkmaid's grip
- spooning sign
- pronator sign
- erythema marginatum
- subcutaneous nodules
- pregnancy or taking oral contraceptive pill
- overcrowded living quarters
- family history of rheumatic fever
- D8/17 B cell antigen positivity
- HLA association
- genetic susceptibility
- indigenous populations; Aboriginal Australian, Asian, and Pacific Islanders
1st investigations to order
- erythrocyte sedimentation rate (ESR)
- WBC count
- blood cultures
- chest x-ray
- throat culture
- rapid antigen test for group A streptococci
- anti-streptococcal serology
- rapid molecular test
monoarthritis in unconfirmed rheumatic fever
possible rheumatic fever
confirmed rheumatic fever
all patients following acute treatment
Rachel Webb, MbChB, MPH & THM, FRACP
Senior Lecturer in Paediatrics
University of Auckland
Paediatric Infectious Diseases Specialist
Starship Children's Hospital and Middlemore Hospital
RW declares that she has no competing interests; she is an active researcher and clinician in acute rheumatic fever/rheumatic heart disease and is a co-investigator on a (non-industry) grant funded by the Health Research Council of New Zealand and gives educational talks and has prepared manuscripts on rheumatic fever solely in capacity as a University of Auckland academic and Paediatric Infectious Diseases Specialist.
Dr Rachel Webb would like to gratefully acknowledge Dr Andrew C. Steer and Dr Jonathan Carapetis, previous contributors to this topic.
ACS and JC declare that they have no competing interests.
Salah Zaher, MD
Professor of Pediatrics
Division of Pediatric Cardiology
Faculty of Medicine
University of Alexandria
El Shatby Children's Hospital
SZ declares that she has no competing interests.
Nigel Wilson, FRACP
Paediatric Cardiologist/Interventional Cardiologist
Paediatric and Congenital Cardiac Services
Green Lane Clinical Services
Starship Children's Hospital
NW declares that he has no competing interests.
Andrea Summer, MD
Assistant Professor of Pediatrics
Medical University of South Carolina
AS declares that she has no competing interests.
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- Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd ed)
- Group A streptococcal infections: guidance and data
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